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Tay-Sachs, Canavan, GM1 and Sandhoff diseases

N-acetyl-a-acid (NAA)

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N-acetyl-a-acid (NAA)

Aspartoacylase breaks down NAA into aspartic acid (an amino acid used in making proteins) and acetic acid. The cycle of production and breakdown of NAA appears to be critical for maintaining the brain's white matter, which consists of nerve fibers covered by myelin. NAA is likely essential for making certain fats (lipids) that are used to produce myelin.

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